OPA1 Controls Apoptotic Cristae Remodeling Independently from Mitochondrial Fusion
Christian Frezza,Sara Cipolat,Olga Martins de Brito,Massimo Micaroni,Galina V. Beznoussenko,Tomasz Rudka,Davide Bartoli,Roman S. Polishuck,Nika N. Danial,Bart De Strooper,Luca Scorrano +10 more
TLDR
Evidence is provided that Optic Atrophy 1 (OPA1), a profusion dynamin-related protein of the inner mitochondrial membrane mutated in dominant optic atrophy, protects from apoptosis by preventing cytochrome c release independently from mitochondrial fusion.About:
This article is published in Cell.The article was published on 2006-07-14 and is currently open access. It has received 1444 citations till now. The article focuses on the topics: Optic Atrophy 1 & Inner mitochondrial membrane.read more
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Phosphorylation and cleavage of presenilin-associated rhomboid-like protein (PARL) promotes changes in mitochondrial morphology.
Danny V. Jeyaraju,Liqun Xu,Marie-Claude Letellier,Sirisha Bandaru,Rodolfo Zunino,Eric A. Berg,Heidi M. McBride,Luca Pellegrini +7 more
TL;DR: It is shown that phosphorylation and cleavage of the vertebrate-specific Pβ domain of the mammalian presenilin-associated rhomboid-like (PARL) protease can influence mitochondrial morphology, providing a blueprint to study the molecular evolution of mitochondrial morphology.
Journal ArticleDOI
Eight human OPA1 isoforms, long and short: What are they for?
TL;DR: The OPA1 protein is a dynamin-related GTPase that controls mitochondrial dynamics, cristae integrity, energetics, and mtDNA maintenance as mentioned in this paper, and the exceptional complexity of this protein is determined by the presence, in humans, of eight different isoforms that, in turn, are proteolytically cleaved into combinations of membrane-anchored long forms and soluble short forms.
Journal ArticleDOI
Fatal infantile mitochondrial encephalomyopathy, hypertrophic cardiomyopathy and optic atrophy associated with a homozygous OPA1 mutation
Ronen Spiegel,Ann Saada,Padraig J. Flannery,Florence Burté,Devorah Soiferman,Morad Khayat,Verónica Eisner,Eugene Vladovski,Robert W. Taylor,Laurence A. Bindoff,Avraham Shaag,Hanna Mandel,Ora Schuler-Furman,Stavit A. Shalev,Orly Elpeleg,Patrick Yu-Wai-Man +15 more
TL;DR: A causal link between a pathogenic homozygous OPA1 mutation and human disease is established for the first time, and the vital role played by OPA2 in mitochondrial biogenesis and mtDNA maintenance is emphasised.
Journal ArticleDOI
TMEM126A, encoding a mitochondrial protein, is mutated in autosomal-recessive nonsyndromic optic atrophy.
Sylvain Hanein,Isabelle Perrault,Olivier Roche,Sylvie Gerber,Noman Khadom,Marlène Rio,Nathalie Boddaert,Marc Jeanpierre,Nora Brahimi,Valérie Serre,Valérie Serre,Dominique Chretien,Nathalie Delphin,Lucas Fares-Taie,Sahran Lachheb,Agnès Rötig,Françoise Meire,Arnold Munnich,Jean-Louis Dufier,Josseline Kaplan,Jean-Michel Rozet +20 more
TL;DR: The first gene, to the authors' knowledge, responsible for this condition, TMEM126A, is identified in a large multiplex inbred Algerian family and subsequently in three other families originating from the Maghreb, supporting the view that mitochondrial dysfunction may be a hallmark of inherited optic neuropathies including isolated autosomal-recessive forms.
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Mitochondrial oxidative phosphorylation and energetic status are reflected by morphology of mitochondrial network in INS-1E and HEP-G2 cells viewed by 4Pi microscopy
TL;DR: Previously undescribed details for shapes upon mitochondrial disintegration are revealed and it is clearly demonstrated that high resolution 3D microscopy is required for visualization of mitochondrial network.
References
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Proapoptotic BAX and BAK: A Requisite Gateway to Mitochondrial Dysfunction and Death
Michael C. Wei,Michael C. Wei,Wei-Xing Zong,Emily H. Cheng,Tullia Lindsten,Vily Panoutsakopoulou,Andrea J. Ross,Kevin A. Roth,Grant R. MacGregor,Craig B. Thompson,Stanley J. Korsmeyer +10 more
TL;DR: In this article, the authors found that doubly deficient cells are resistant to multiple apoptotic stimuli that act through disruption of mitochondrial function: staurosporine, ultraviolet radiation, growth factor deprivation, etoposide, and the endoplasmic reticulum stress stimuli thapsigargin and tunicamycin.
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TL;DR: The therapeutic induction of MOMP may restore apoptosis in cancer cells in which it is disabled, and the general rules governing the pathophysiology and controversial issues regarding its regulation are discussed.
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Two CD95 (APO-1/Fas) signaling pathways
Carsten Scaffidi,Simone Fulda,Anu Srinivasan,Claudia Friesen,Feng Li,Kevin J. Tomaselli,Klaus-Michael Debatin,Peter H. Krammer,Marcus E. Peter +8 more
TL;DR: In the presence of caspase‐3 the amount of active casp enzyme‐8 generated at the DISC determines whether a mitochondria‐independent apoptosis pathway is used (type I cells) or not (type II cells).
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The expanding role of mitochondria in apoptosis
TL;DR: The complexity of the apoptotic program began to increase with the discovery of Bcl-2, a gene whose product causes resistance to apoptosis in lymphocytes, and the complex role of mitochondria in apoptosis came into focus when biochemical studies identified several mitochondrial proteins that are able to activate cellular apoptotic programs directly.
Journal ArticleDOI
Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development
TL;DR: It is concluded that Mfn1 and Mfn2 have both redundant and distinct functions and act in three separate molecular complexes to promote mitochondrial fusion, and by enabling cooperation between mitochondria, has protective effects on the mitochondrial population.